Background

Increasingly data suggest that human papillomavirus (HPV) may be a cause of some vulvar
malignancies.

Frequency

Vulvar cancer accounts for approximately 5% of all female genital malignancies. It occurs in
about 1.5 per 100,000 women-years in developed countries but is 2-3 times more frequent in
underdeveloped countries. With the exception of the rare sarcomas, this cancer appears most
frequently in women aged 65-75 years, and, in some series, almost half of the patients are
aged 70 years or older. Vulvar cancer can appear in younger patients, and, in some large
cancer referral institutions, approximately 15% of all vulvar cancers occur in women younger
than 40 years. These young patients tend to have early microcarcinomas, which may be
associated with diffuse intraepithelial neoplasia of the vulva.

As the population ages, the incidence of vulvar cancer may be increasing slowly. In a recent
annual report, almost 50% of patients reported to have vulvar cancer were aged 70 years or
older, with 15% aged 80 years or older. In most series, an extended delay in diagnosis
appears to occur mainly because the patient does not seek medical attention for many months
or because the lesion is treated medically for months, without biopsy for definitive diagnosis.

Diagnosis

Histological evaluation is a prerequisite before planning definitive therapy for changes in the
epithelium of the vulva, whether pigmentation, hypertrophy, or lump or mass occurs. Many
techniques can be used in the office setting to obtain adequate tissue for pathological
evaluation. A dermal punch biopsy can be used as it is used elsewhere on the skin. If a lesion
is small, excision may not only be diagnostic but also therapeutic. A local anesthetic is
usually sufficient, and sutures are placed as needed.

Most vulvar cancer is squamous in origin. Because the vulva is covered with skin, any
malignancy that appears elsewhere on the skin also can occur on the vulva. Melanoma is the
second most frequent histological type, but this represents less than 5% of vulvar cancers.

Squamous vulvar cancer can have many different growth characteristics. It can occur in an
area of epithelial neoplasia that develops into a small nodule, which may break down and
ulcerate. Small, warty, or cauliflowerlike growths may arise and be confused with condyloma
acuminata. Squamous carcinomas can appear in a background of atrophic changes (ie, lichen
sclerosis) or in hypertrophic epithelium. Long-term pruritus, lumps, or masses on the vulva
are present in most patients with invasive vulvar cancer.

Metastasis

The cancer can appear anywhere on the vulva, although about three fourths arise primarily on
the labia. The more rare types, such as Bartholin gland carcinomas, tend to be localized to
that specific region. Because the vulva is rich in lymphatics, metastasis to the inguinal lymph
node can occur early in the process. Lymph node involvement is directly related to the depth
of stromal invasion, as well as to the size of the primary lesion. Fortunately, bilateral inguinal
nodal involvement without ipsilateral side involvement is unusual. This has therapeutic
indications. The disease is usually localized and well demarcated; however, in advanced
disease, determining the exact site of origin is impossible. Multifocal patterns with invasive
cancer are unusual, although kissing lesions can occur as isolated lesions. Unilateral lesions
appear to be the norm, particularly in postmenopausal patients.

The clinical evaluation of possible inguinal lymph node metastasis is very imprecise. In 1988,
the International Federation of Gynecology and Obstetrics (FIGO) adopted a surgical staging
system based on the primary tumor, regional lymph node, remote metastases (TNM)
classification system. In 2008, FIGO revised the staging of vulvar cancer.[1, 2]

Table 1. Carcinoma of the Vulva: FIGO nomenclature (Open Table in a new window)

Stage Characteristics
I Tumor confined to the vulva
IA Lesions ≤2 cm in size, confined to the vulva or perineum and with stromal invasion <
1.0 mm*, no nodal metastasis
IB Lesions >2 cm in size or with stromal invasion >1.0 mm*, confined to the vulva or
perineum with negative nodes
II Tumor of any size with extension to adjacent perineal structures (1/3 lower urethra, 1/3
lower vagina, anus) with negative nodes
III Tumor of any size with or without extension to adjacent perineal structures (1/3 lower
urethra, 1/3 lower vagina, anus) with positive inguinofemoral lymph nodes
IIIA (i) With 1 lymph node metastasis (≥5 mm), or

(ii) 1-2 lymph node metastasis(es) (< 5 mm)

IIIB (i) With 2 or more lymph node metastases (≥5 mm), or

(ii) 3 or more lymph node metastases (< 5 mm)

IIICWith positive nodes with extracapsular spread

IV Tumor invades other regional (2/3 upper urethra, 2/3 upper vagina), or distant
structures
IVA Tumor invades any of the following:

(i) Upper urethral and/or vaginal mucosa, bladder mucosa, rectal mucosa, or fixed to
pelvic bone, or
 (ii) Fixed or ulcerated inguinofemoral lymph nodes

IVB Any distant metastasis including pelvic lymph nodes

*The depth of invasion is defined as the measurement of the tumor from the epithelial-
stromal junction of the adjacent, most superficial dermal papilla to the deepest point of
invasion.

Treatment

A small primary lesion on the vulva (ie, < 2 cm) with superficial invasion (ie, < 1 mm from
the epithelial stromal junction of the adjacent, most superficial dermal papillae) has
essentially no risk of lymph node metastasis. Consequently, these lesions can be treated with
wide local excision, ensuring that adequate surgical margins are present (not only on the skin
but also deep margins).

In a review of randomized controlled trials that assessed medical interventions in adult

women diagnosed with high-grade vulval intraepithelial neoplasia (VIN), imiquimod appears
to be effective; however, but its safety needs further examination. Indole-3-carbinol also
appears to be safe but may not be as effective as imiquimod or surgery.[3]

In larger lesions (ie, stage IB or greater or with stromal invasion >1 mm), the incidence of
ipsilateral inguinal lymph node involvement increases as the depth of invasion, as well as the
gross size, increases. Consequently, inguinal lymphadenectomy is part of the primary surgical
procedure. This can be performed through a small separate inguinal incision, removing the
lymph nodes above the cribriform fascia and in the opening of the fascia at the fossa ovalis. If
the results are negative on frozen section of these lymph nodes, then a modified partial
vulvectomy is the only treatment necessary. If the results on frozen section of the ipsilateral
lymph nodes are positive, then most physicians suggest removing the lymph nodes on the
contralateral inguinal area as well.

The lesion itself can be treated conservatively, with a partial vulvectomy. Performing
complete vulvectomy is an outdated treatment unless the cancer is present bilaterally. If
clitoral involvement is present, lymphatic drainage can be direct to the pelvic lymph nodes.
Studies have demonstrated that, even with clitoral involvement, the deep lymph nodes are not
involved unless the inguinal nodes have evidence of metastasis also. Pelvic
lymphadenectomy is largely discontinued, even in cases of lymph node involvement. A large,
prospective, randomized study conducted by the Gynecologic Oncology Group (GOG) noted
that patient survival rates are better if the pelvic and inguinal area is treated with radiation
postsurgically, as compared to patients treated with pelvic lymphadenectomies, even when
the pelvic nodes are not involved.[4] Incidence of lymph node metastasis seems to be
increased if vascular lymphatic space is involved.

In women with locally advanced vulvar cancer, no significant differences in survival or

adverse events were found when primary chemoradiation or neoadjuvant chemoradiation
were compared with primary surgery. However, these findings were based on small numbers
and few studies in this Cochrane review.[5] Good, quality studies that compare primary
treatments in locally advanced vulvar cancer are needed.

Prognosis

Contemporary data suggest that the overall 5-year survival rate of patients with stage I
epidermoid invasive cancer is 85-90%. The survival rate decreases with increasing stage;
however, an approximate 5-year survival rate of 40% can be obtained, even in patients with
lymph node metastasis.

In a review of the National Cancer Data Base, patients with positive inguinal lymph nodes
were found to have 5-year survival rates of 64% with 2-cm lesions and 43% with lesions
greater than 2 cm. In patients with primary lesions of any size, the survival rate was identical
whether 1 lymph node was positive or 2-3 lymph nodes were positive (55% vs 59%). The
survival rate in patients with 4 or more positive nodes was only 33% at 5 years. The 5-year
survival rate of patients with 1 positive node, without radiation, was 68% and was 56% with
radiation. If 2 or more lymph nodes were positive, the survival rate was 46% without
radiation therapy and 48% with radiation.[6]

The 2006 Annual Report noted a 5-year survival rate of 82% in patients with negative lymph
nodes who were treated with surgery only, compared to 72% for patients treated with surgery
and radiation. If lymph nodes were positive, the survival rate was 48% in patients treated
with surgery alone, compared to 31% in patients treated with surgery plus radiation. These
studies raise the question of whether or not postoperative radiation therapy is as advantageous
as the GOG study noted.

Follow-up

More than 80% of recurrences appear within the first 2 years after therapy, and they may be
either local or distant. Because many reoccurring lesions appear locally and near the site of
the primary lesion, initial close follow-up is necessary. Visual examination provides the best
follow-up.

Local recurrences are more common in patients with large primary tumors than in patients
with metastatic disease in the lymph nodes, and local occurrences can appear when the
margins are clear on the original operative specimen. Local recurrences can be managed
successfully in many instances by repeat local excision and/or interstitial radiation.

Recurrent lesions in the lymph node area, as well as in distant sites, are difficult to treat, and
survival rates are poor. If recurrences appear in the inguinal area, excision with or without
radiation therapy may be beneficial. Distant metastasis are treated most effectively with
chemotherapy, with cisplatin as the drug of choice, and 30% response rates have been
achieved in reports.

Background

Melanoma is the second most common invasive cancer occurring in the vulva, but its
occurrence is rare. Melanoma probably arises from a lesion containing a junctional or
compound nevus. Consider pigmented lesions on the vulva suspicious if they are blue-black
in color, have a jagged or fuzzy border, are raised or ulcerated, or are larger than
approximately 1 cm. Melanomas may be misdiagnosed as undifferentiated squamous
carcinoma, particularly if they are amelanotic. Most melanomas are located on the labia
minora or clitoris, and prognosis is related to the size of the lesion and the depth of invasion.
The Clark classification commonly used for melanoma elsewhere on the skin is of prognostic
benefit for melanoma of the vulva. The Breslow modification expresses invasion in
micrometers compared with location of different skin structures.

Approximately 3% of all melanomas are located in the genital tract. Melanoma of the vulva
accounts for 5-7% of invasive vulvar cancers and has an estimated annual incident rate of 1
per 1,000,000 women. The disease can affect women of all ages (eg, women aged 7-97 y in 1
study) but is more common in the older population, with almost half of the patients aged 70
years or older. More than 90% of melanomas occur in white women.

Treatment [7]

In the past, this lesion was treated with radical vulvectomy and bilateral inguinal
lymphadenectomy. In recent years, more conservative treatment, such as has been practiced
for this lesion elsewhere on the body, has become more common. A radical local excision
with 2-cm margins appears to be adequate for most well-circumscribed lesions. Whether
inguinal lymphadenectomy should be performed for this cancer is undecided at present.
Obviously, if lymph nodes are involved, this finding is not only diagnostic but also
prognostic. If lymph nodes are negative, the patient may be reassured. Lymph node
involvement is directly related to the depth of invasion. If the disease is intraepithelial, the
cure rate is close to 100% and is reported to be as high as 99% with invasion of 1.5 mm or
less. The survival rate drops to 65-70% if the lesion invades 1.5-4 mm.

Medical management for metastatic disease continues to be experimental. If the melanoma

recurs locally in the vulvar area, reexcision may be adequate therapy, with long-term
survival.

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Malignant Vulvar Lesions

Updated: May 17, 2011

 Overview
 Melanoma
 Paget Disease
 Show All

Tables
References

Overview

Background

Increasingly data suggest that human papillomavirus (HPV) may be a cause of some vulvar
malignancies.

Frequency

Vulvar cancer accounts for approximately 5% of all female genital malignancies. It occurs in
about 1.5 per 100,000 women-years in developed countries but is 2-3 times more frequent in
underdeveloped countries. With the exception of the rare sarcomas, this cancer appears most
frequently in women aged 65-75 years, and, in some series, almost half of the patients are
aged 70 years or older. Vulvar cancer can appear in younger patients, and, in some large
cancer referral institutions, approximately 15% of all vulvar cancers occur in women younger
than 40 years. These young patients tend to have early microcarcinomas, which may be
associated with diffuse intraepithelial neoplasia of the vulva.

As the population ages, the incidence of vulvar cancer may be increasing slowly. In a recent
annual report, almost 50% of patients reported to have vulvar cancer were aged 70 years or
older, with 15% aged 80 years or older. In most series, an extended delay in diagnosis
appears to occur mainly because the patient does not seek medical attention for many months
or because the lesion is treated medically for months, without biopsy for definitive diagnosis.

Diagnosis
Histological evaluation is a prerequisite before planning definitive therapy for changes in the
epithelium of the vulva, whether pigmentation, hypertrophy, or lump or mass occurs. Many
techniques can be used in the office setting to obtain adequate tissue for pathological
evaluation. A dermal punch biopsy can be used as it is used elsewhere on the skin. If a lesion
is small, excision may not only be diagnostic but also therapeutic. A local anesthetic is
usually sufficient, and sutures are placed as needed.

Most vulvar cancer is squamous in origin. Because the vulva is covered with skin, any
malignancy that appears elsewhere on the skin also can occur on the vulva. Melanoma is the
second most frequent histological type, but this represents less than 5% of vulvar cancers.

Squamous vulvar cancer can have many different growth characteristics. It can occur in an
area of epithelial neoplasia that develops into a small nodule, which may break down and
ulcerate. Small, warty, or cauliflowerlike growths may arise and be confused with condyloma
acuminata. Squamous carcinomas can appear in a background of atrophic changes (ie, lichen
sclerosis) or in hypertrophic epithelium. Long-term pruritus, lumps, or masses on the vulva
are present in most patients with invasive vulvar cancer.

Metastasis

The cancer can appear anywhere on the vulva, although about three fourths arise primarily on
the labia. The more rare types, such as Bartholin gland carcinomas, tend to be localized to
that specific region. Because the vulva is rich in lymphatics, metastasis to the inguinal lymph
node can occur early in the process. Lymph node involvement is directly related to the depth
of stromal invasion, as well as to the size of the primary lesion. Fortunately, bilateral inguinal
nodal involvement without ipsilateral side involvement is unusual. This has therapeutic
indications. The disease is usually localized and well demarcated; however, in advanced
disease, determining the exact site of origin is impossible. Multifocal patterns with invasive
cancer are unusual, although kissing lesions can occur as isolated lesions. Unilateral lesions
appear to be the norm, particularly in postmenopausal patients.

The clinical evaluation of possible inguinal lymph node metastasis is very imprecise. In 1988,
the International Federation of Gynecology and Obstetrics (FIGO) adopted a surgical staging
system based on the primary tumor, regional lymph node, remote metastases (TNM)
classification system. In 2008, FIGO revised the staging of vulvar cancer.[1, 2]

Table 1. Carcinoma of the Vulva: FIGO nomenclature (Open Table in a new window)

Stage Characteristics

I Tumor confined to the vulva

IA Lesions ≤2 cm in size, confined to the vulva or perineum and with stromal invasion < 1.0 mm*,
no nodal metastasis

IB Lesions >2 cm in size or with stromal invasion >1.0 mm*, confined to the vulva or perineum
with negative nodes
II Tumor of any size with extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower
vagina, anus) with negative nodes

III Tumor of any size with or without extension to adjacent perineal structures (1/3 lower
urethra, 1/3 lower vagina, anus) with positive inguinofemoral lymph nodes

IIIA (i) With 1 lymph node metastasis (≥5 mm), or

(ii) 1-2 lymph node metastasis(es) (< 5 mm)

IIIB (i) With 2 or more lymph node metastases (≥5 mm), or

(ii) 3 or more lymph node metastases (< 5 mm)

IIIC With positive nodes with extracapsular spread

IV Tumor invades other regional (2/3 upper urethra, 2/3 upper vagina), or distant structures

IVA Tumor invades any of the following:

(i) Upper urethral and/or vaginal mucosa, bladder mucosa, rectal mucosa, or fixed to pelvic
bone, or

(ii) Fixed or ulcerated inguinofemoral lymph nodes

IVB Any distant metastasis including pelvic lymph nodes

*The depth of invasion is defined as the measurement of the tumor from the epithelial-stromal
junction of the adjacent, most superficial dermal papilla to the deepest point of invasion.
Treatment

A small primary lesion on the vulva (ie, < 2 cm) with superficial invasion (ie, < 1 mm from
the epithelial stromal junction of the adjacent, most superficial dermal papillae) has
essentially no risk of lymph node metastasis. Consequently, these lesions can be treated with
wide local excision, ensuring that adequate surgical margins are present (not only on the skin
but also deep margins).

In a review of randomized controlled trials that assessed medical interventions in adult

women diagnosed with high-grade vulval intraepithelial neoplasia (VIN), imiquimod appears
to be effective; however, but its safety needs further examination. Indole-3-carbinol also
appears to be safe but may not be as effective as imiquimod or surgery.[3]

In larger lesions (ie, stage IB or greater or with stromal invasion >1 mm), the incidence of
ipsilateral inguinal lymph node involvement increases as the depth of invasion, as well as the
gross size, increases. Consequently, inguinal lymphadenectomy is part of the primary surgical
procedure. This can be performed through a small separate inguinal incision, removing the
lymph nodes above the cribriform fascia and in the opening of the fascia at the fossa ovalis. If
the results are negative on frozen section of these lymph nodes, then a modified partial
vulvectomy is the only treatment necessary. If the results on frozen section of the ipsilateral
lymph nodes are positive, then most physicians suggest removing the lymph nodes on the
contralateral inguinal area as well.

The lesion itself can be treated conservatively, with a partial vulvectomy. Performing
complete vulvectomy is an outdated treatment unless the cancer is present bilaterally. If
clitoral involvement is present, lymphatic drainage can be direct to the pelvic lymph nodes.
Studies have demonstrated that, even with clitoral involvement, the deep lymph nodes are not
involved unless the inguinal nodes have evidence of metastasis also. Pelvic
lymphadenectomy is largely discontinued, even in cases of lymph node involvement. A large,
prospective, randomized study conducted by the Gynecologic Oncology Group (GOG) noted
that patient survival rates are better if the pelvic and inguinal area is treated with radiation
postsurgically, as compared to patients treated with pelvic lymphadenectomies, even when
the pelvic nodes are not involved.[4] Incidence of lymph node metastasis seems to be
increased if vascular lymphatic space is involved.

In women with locally advanced vulvar cancer, no significant differences in survival or

adverse events were found when primary chemoradiation or neoadjuvant chemoradiation
were compared with primary surgery. However, these findings were based on small numbers
and few studies in this Cochrane review.[5] Good, quality studies that compare primary
treatments in locally advanced vulvar cancer are needed.

Prognosis

Contemporary data suggest that the overall 5-year survival rate of patients with stage I
epidermoid invasive cancer is 85-90%. The survival rate decreases with increasing stage;
however, an approximate 5-year survival rate of 40% can be obtained, even in patients with
lymph node metastasis.

In a review of the National Cancer Data Base, patients with positive inguinal lymph nodes
were found to have 5-year survival rates of 64% with 2-cm lesions and 43% with lesions
greater than 2 cm. In patients with primary lesions of any size, the survival rate was identical
whether 1 lymph node was positive or 2-3 lymph nodes were positive (55% vs 59%). The
survival rate in patients with 4 or more positive nodes was only 33% at 5 years. The 5-year
survival rate of patients with 1 positive node, without radiation, was 68% and was 56% with
radiation. If 2 or more lymph nodes were positive, the survival rate was 46% without
radiation therapy and 48% with radiation.[6]

The 2006 Annual Report noted a 5-year survival rate of 82% in patients with negative lymph
nodes who were treated with surgery only, compared to 72% for patients treated with surgery
and radiation. If lymph nodes were positive, the survival rate was 48% in patients treated
with surgery alone, compared to 31% in patients treated with surgery plus radiation. These
studies raise the question of whether or not postoperative radiation therapy is as advantageous
as the GOG study noted.

Follow-up

More than 80% of recurrences appear within the first 2 years after therapy, and they may be
either local or distant. Because many reoccurring lesions appear locally and near the site of
the primary lesion, initial close follow-up is necessary. Visual examination provides the best
follow-up.

Local recurrences are more common in patients with large primary tumors than in patients
with metastatic disease in the lymph nodes, and local occurrences can appear when the
margins are clear on the original operative specimen. Local recurrences can be managed
successfully in many instances by repeat local excision and/or interstitial radiation.

Recurrent lesions in the lymph node area, as well as in distant sites, are difficult to treat, and
survival rates are poor. If recurrences appear in the inguinal area, excision with or without
radiation therapy may be beneficial. Distant metastasis are treated most effectively with
chemotherapy, with cisplatin as the drug of choice, and 30% response rates have been
achieved in reports.

Melanoma

Background

Melanoma is the second most common invasive cancer occurring in the vulva, but its
occurrence is rare. Melanoma probably arises from a lesion containing a junctional or
compound nevus. Consider pigmented lesions on the vulva suspicious if they are blue-black
in color, have a jagged or fuzzy border, are raised or ulcerated, or are larger than
approximately 1 cm. Melanomas may be misdiagnosed as undifferentiated squamous
carcinoma, particularly if they are amelanotic. Most melanomas are located on the labia
minora or clitoris, and prognosis is related to the size of the lesion and the depth of invasion.
The Clark classification commonly used for melanoma elsewhere on the skin is of prognostic
benefit for melanoma of the vulva. The Breslow modification expresses invasion in
micrometers compared with location of different skin structures.

Approximately 3% of all melanomas are located in the genital tract. Melanoma of the vulva
accounts for 5-7% of invasive vulvar cancers and has an estimated annual incident rate of 1
per 1,000,000 women. The disease can affect women of all ages (eg, women aged 7-97 y in 1
study) but is more common in the older population, with almost half of the patients aged 70
years or older. More than 90% of melanomas occur in white women.

Treatment [7]

In the past, this lesion was treated with radical vulvectomy and bilateral inguinal
lymphadenectomy. In recent years, more conservative treatment, such as has been practiced
for this lesion elsewhere on the body, has become more common. A radical local excision
with 2-cm margins appears to be adequate for most well-circumscribed lesions. Whether
inguinal lymphadenectomy should be performed for this cancer is undecided at present.
Obviously, if lymph nodes are involved, this finding is not only diagnostic but also
prognostic. If lymph nodes are negative, the patient may be reassured. Lymph node
involvement is directly related to the depth of invasion. If the disease is intraepithelial, the
cure rate is close to 100% and is reported to be as high as 99% with invasion of 1.5 mm or
less. The survival rate drops to 65-70% if the lesion invades 1.5-4 mm.

Medical management for metastatic disease continues to be experimental. If the melanoma

recurs locally in the vulvar area, reexcision may be adequate therapy, with long-term
survival.

Paget Disease

Background

Paget disease of the vulva is rare. It occurs in women in the seventh decade of life but can
occur in young patients. Symptoms of pruritus and tenderness or identification of a vulvar
lesion occur most frequently. The patient may experience symptoms for several years before
seeking medical advice. The lesion may be localized to one labium or involve the entire
vulvar epithelium. It may extend to the perirectal area, buttocks, inguinal area, or mons. It has
been observed extending into the vagina. Because of its rarity, frequency data are
unavailable.

Clinical and histological features

Vulvar lesions usually are hyperemic, and they may be demarcated sharply and thickened
with foci of excoriation and induration. The vulvar skin may be thick, leading to the
impression of leukoplakia with the cake icing effect. This classic finding almost is
pathognomonic for Paget disease. The lesion is usually superficial and is considered an
intraepithelial lesion, although an underlying adenocarcinoma may be associated with Paget
disease. Older literature suggests that underlying adenocarcinoma occurs in about a quarter of
cases, but more recent data find less association of Paget disease with an underlying
adenocarcinoma.

Obtain adequate biopsies to make an accurate diagnosis. Histologically, it commonly presents

with large cells of clear cytoplasm in a heavy lymphocytic infiltration in the dermis; it can be
confused with amelanotic melanoma. If any thickened indurated area is present, then obtain
adequate deep biopsy in order to rule out adenocarcinoma.
Treatment [8, 9]

If only intraepithelial Paget disease is present, wide local excision is adequate treatment.
Histological evaluation of the epithelium extends, in many instances, far beyond the visual
limits of the lesion, and, therefore, wide adequate margins are necessary to remove the lesion.
If an underlying adenocarcinoma is present, then treat the lesion as invasive squamous cell
carcinoma is treated. If tumor cells are present at the margin of the excision, then recurrence
can be quite high. Some investigators obtain frozen section of the margins and, if positive,
continue to obtain wider margins. Unfortunately, even with negative margins, recurrences are
possible, and new lesions can be treated in the same manner as the primary disease (ie, wide
local excision). These may occur years after diagnosis of the primary lesion.

Currently, intraepithelial Paget disease and Paget disease with an underlying adenocarcinoma
are thought to be 2 separate entities. The cases of patients who were diagnosed with Paget
disease and who refused primary treatment have been followed for over a decade without
development of an underlying adenocarcinoma. As with other vulvar lesions, frequent visual
examinations are necessary in order to determine disease-free status.

Table 1. Carcinoma of the Vulva: FIGO nomenclature

Stage Characteristics
I Tumor confined to the vulva
IA Lesions ≤2 cm in size, confined to the vulva or perineum and with stromal invasion <
1.0 mm*, no nodal metastasis
IB Lesions >2 cm in size or with stromal invasion >1.0 mm*, confined to the vulva or
perineum with negative nodes
II Tumor of any size with extension to adjacent perineal structures (1/3 lower urethra, 1/3
lower vagina, anus) with negative nodes
III Tumor of any size with or without extension to adjacent perineal structures (1/3 lower
urethra, 1/3 lower vagina, anus) with positive inguinofemoral lymph nodes
IIIA (i) With 1 lymph node metastasis (≥5 mm), or

(ii) 1-2 lymph node metastasis(es) (< 5 mm)

IIIB (i) With 2 or more lymph node metastases (≥5 mm), or

(ii) 3 or more lymph node metastases (< 5 mm)

IIIC With positive nodes with extracapsular spread

IV Tumor invades other regional (2/3 upper urethra, 2/3 upper vagina), or distant
 structures
IVA Tumor invades any of the following:

(i) Upper urethral and/or vaginal mucosa, bladder mucosa, rectal mucosa, or fixed to
pelvic bone, or

(ii) Fixed or ulcerated inguinofemoral lymph nodes

IVB Any distant metastasis including pelvic lymph nodes

*The depth of invasion is defined as the measurement of the tumor from the epithelial-
stromal junction of the adjacent, most superficial dermal papilla to the deepest point of
invasion.

Table 1. Carcinoma of the Vulva: FIGO nomenclature

Stage Characteristics

I Tumor confined to the vulva

IA Lesions ≤2 cm in size, confined to the vulva or perineum and with stromal invasion < 1.0 mm*,
no nodal metastasis

IB Lesions >2 cm in size or with stromal invasion >1.0 mm*, confined to the vulva or perineum
with negative nodes

II Tumor of any size with extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower
vagina, anus) with negative nodes

III Tumor of any size with or without extension to adjacent perineal structures (1/3 lower
urethra, 1/3 lower vagina, anus) with positive inguinofemoral lymph nodes

IIIA (i) With 1 lymph node metastasis (≥5 mm), or

(ii) 1-2 lymph node metastasis(es) (< 5 mm)

IIIB (i) With 2 or more lymph node metastases (≥5 mm), or

 (ii) 3 or more lymph node metastases (< 5 mm)

IIIC With positive nodes with extracapsular spread

IV Tumor invades other regional (2/3 upper urethra, 2/3 upper vagina), or distant structures

IVA Tumor invades any of the following:

(i) Upper urethral and/or vaginal mucosa, bladder mucosa, rectal mucosa, or fixed to pelvic
bone, or

(ii) Fixed or ulcerated inguinofemoral lymph nodes

IVB Any distant metastasis including pelvic lymph nodes

*The depth of invasion is defined as the measurement of the tumor from the epithelial-stromal
junction of the adjacent, most superficial dermal papilla to the deepest point of invasion.

Table 1 of 1

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